Factor XIII: novel structural and functional aspects
نویسندگان
چکیده
منابع مشابه
Novel aspects of platelet factor XIII function.
Pools of factor XIII (FXIII) exist in the plasma and within the cytoplasm of hematopoietic cells, including platelets. The functions of the cellular form, FXIII-A, have been assumed to be intracellular in nature, as the protein lacks a signal sequence for its release. Mounting evidence now suggests that platelet FXIII-A modulates hemostasis by several different mechanisms. In this condensed rev...
متن کاملNovel aspects of factor XIII deficiency.
PURPOSE OF REVIEW Here we review recent developments concerning the diagnosis, classification and treatment of factor XIII (FXIII) deficiency and new findings related to the pathogenesis of the disease. RECENT FINDINGS Most recently, the International Society on Thrombosis and Haemostasis, Scientific and Standardization Committee published a guideline for the diagnosis and classification of F...
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Congenital factor XIII (FXIII) deficiency is a rare, autosomal-recessive disorder, with most patients having an A-subunit (FXIII-A) deficiency. Patients experience life-threatening bleeds, impaired wound healing, and spontaneous abortions. In many countries, only plasma or cryoprecipitate treatments are available, but these carry a risk for allergic reactions and infection with blood-borne path...
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The coagulation factor XIII(FXIII) is a plasma circulating heterotetrameric protransglutaminase that acts at the end of the coagulation cascade by covalently cross-linking preformed fibrin clots (to themselves and to fibrinolytic inhibitors) in order to stabilize them against fibrinolysis. It circulates in the plasma as a heterotetramer composed of two homomeric catalytic Factor XIIIA2 (FXIIIA2...
متن کاملRecombinant factor XIII (rFXIII): a novel treatment of congenital factor XIII deficiency
2015 Factor XIII (FXIII) is composed of two catalytic A subunits and two carrier B subunits. Following activation by thrombin FXIII becomes plasma transglutaminase, which crosslinks the γ-glutamyl-ɛ-lysine residues of fibrin chains stabilizing fibrin clot. Congenital deficiency of factor XIII results in a severe life-long hemorrhagic disorder, abnormal wound healing in about 30% of patients and...
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ژورنال
عنوان ژورنال: Journal of Thrombosis and Haemostasis
سال: 2011
ISSN: 1538-7933
DOI: 10.1111/j.1538-7836.2010.04070.x